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Conflict of Interest Case 1 7/1/2013 2013 Internal Medicine Board Review Dermatology Lindy Fox, MD Department of Dermatology University of California, San Francisco 1 Conflict of Interest I have no relevant conflicts to disclose. 2 Case 1 • A 45 year old man presents with painless vesicles and bullae on his face and dorsal hands 3 1 7/1/2013 • You also notice – Erosions (fragility) – Hypertrichosis – Hyperpigmentation – Milia 4 Case 1, Question 1 The most likely diagnosis is: A. Pemphigus vulgaris B. Bullous impetigo C. Bullous pemphigoid D. Porphyria cutanea tarda E. Dermatitis herpetiformis 5 Case 1, Question 1 The most likely diagnosis is: A. Pemphigus vulgaris B. Bullous impetigo C. Bullous pemphigoid D. Porphyria cutanea tarda E. Dermatitis herpetiformis 6 2 7/1/2013 Case 1, Question 2 Porphyria cutanea tarda The underlying condition most likely to be associated is: A. Hemochromatosis B. Hepatitis C C. Chronic renal insufficiency D. Diabetes mellitus E. NSAID use 7 Case 1, Question 2 Porphyria cutanea tarda The underlying condition most likely to be associated is: A. Hemochromatosis B. Hepatitis C C. Chronic renal insufficiency D. Diabetes mellitus E. NSAID use 8 Porphyria Cutanea Tarda (PCT) • Most common form of porphyria • Presents in 5th decade of life • M (60%), F (40%) •Risk factors – HCV 85% – Hemochromatosis – Alcoholism – Genetic predisposition • Iron overload leads to reduced uroporphyrinogen decarboxylase activity 9 3 7/1/2013 Porphyria Cutanea Tarda • Sun-exposed sites (dorsal hands, ears, face) • Non-inflammatory bulla • Skin fragility • Facial hypertrichosis • Milia • Hyperpigmentation 10 Porphyria Cutanea Tarda Treatment • Phlebotomy +/- erythropoetin • Low dose hydroxychloroquine – 200 mg twice per week • Sun avoidance/photoprotection 11 Case 2 • 43 yo Scandinavian male • Chronic abdominal pain, diarrhea, weight loss • Small bowel biopsy: shortening of intestinal villa • Pruritic papules and vesicles on extensor surfaces and buttocks • No mucous membrane involvement 12 4 7/1/2013 13 Case 2, Question 1 The most likely diagnosis is: A. Pemphigus vulgaris B. Bullous impetigo C. Bullous pemphigoid D. Porphyria cutanea tarda E. Dermatitis herpetiformis 14 Case 2, Question 1 The most likely diagnosis is: A. Pemphigus vulgaris B. Bullous impetigo C. Bullous pemphigoid D. Porphyria cutanea tarda E. Dermatitis herpetiformis 15 5 7/1/2013 Case 2, Question 2 Dermatitis Herpetiformis This condition is most closely associated with: A. Underlying lymphoma B. Gluten-sensitive enteropathy C. Autoimmune diseases D. Diabetes mellitus E. No associated underlying condition 16 Case 2, Question 2 Dermatitis Herpetiformis This condition is most closely associated with: A. Underlying lymphoma B. Gluten-sensitive enteropathy C. Autoimmune diseases D. Diabetes mellitus E. No associated underlying condition 17 Dermatitis Herpetiformis • Symmetric, erythematous vesicles and papules in groups (“herpetiform”) • Intensely pruritic • Distribution is a clue: – Elbows, knees, forearms, buttocks, scalp, neck 18 6 7/1/2013 Dermatitis Herpetiformis Associated Diseases • Associated with gluten-sensitive enteropathy • Increased risk of GI lymphoma • Thyroid diseases in 20% – hypothyroidism #1 – acute autoimmune thyroiditis – hyperthyroidism • Other: pernicious anemia, Addison’s disease 19 Dermatitis Herpetiformis Diagnosis Test Mode Result in DH Notes Skin biopsy H&E Collections of DIF(+) DIF neutrophils at granular IgA upper dermal-epidermal dermis junction IgA tissue ELISA, blood Sensitivity 90% Higher false (+), transglutaminase Specificity 95% confirm with anti- endomysial Ab IgA anti- IF, blood (+) 70-90% Antigen is tissue endomysial Ab transglutaminase 20 Dermatitis Herpetiformis Treatment • Gluten free diet • Dapsone (50-300 mg daily) – rapid response • Does not respond to topical or systemic steroids 21 7 7/1/2013 Pemphigus Vulgaris • Elderly • Widespread, friable blisters -> erosions • Oral erosions • Paraneoplastic association suggested by significant mucosal disease 22 23 Case 3 • 48 yr old man • Facial rash x 3 months • Increasing fatigue • Difficulty stocking overhead shelves 24 8 7/1/2013 25 26 27 9 7/1/2013 Case 3, Question 1 The lab test most likely to be abnormal is: A. ESR B. Anti-smith antibody C. Rheumatoid factor D. Serum creatine kinase E. Anti-dsDNA 28 Case 3, Question 1 The lab test most likely to be abnormal is: A. ESR B. Anti-smith antibody C. Rheumatoid factor D. Serum creatine kinase E. Anti-dsDNA 29 Dermatomyositis • Proximal muscle weakness • Characteristic skin findings – Heliotrope: peri-orbital edema, violaceous rash @ eyelids – Gottron’s papules: flat, violaceous @ MCP, PIP, DIP joints – Photosensitive rash, shawl sign – Skin biopsy: similar to lupus (vacuolar interface + mucin) • Lab tests: – Elevated CK or aldolase – Muscle biopsy, electromyography – ANA positive in 60-80% – Anti-Jo antibody associated with interstitial lung disease 30 10 7/1/2013 Case 3, Question 2 Dermatomyositis • In an adult female patient with dermatomyositis, which is the most important test to evaluate for an associated malignancy? A. Thyroid scan B. Mammogram C. Colonoscopy D. Upper endoscopy E. Pelvic ultrasound 31 Case 3, Question 2 Dermatomyositis • In an adult female patient with dermatomyositis, which is the most important test to evaluate for an associated malignancy? A. Thyroid scan B. Mammogram C. Colonoscopy D. Upper endoscopy E. Pelvic ultrasound 32 Dermatomyositis Paraneoplastic Associations • Dermatomyositis is associated with underlying malignancy in 32% of adult patients – Risk highest > age 45, especially men • Women: ovarian cancer • Men: lung cancer • Asians: hepatomas, esophageal adenoCA 33 11 7/1/2013 Case 4 • Healthy 20 yo college student • Pruritic eruption x 10 days 34 Case 4, Question 1 The most likely diagnosis is: A. Psoriasis B. Pityriasis rosea C. Secondary syphilis D. Subacute cutaneous lupus E. Tinea versicolor 35 Case 4, Question 1 The most likely diagnosis is: A. Psoriasis B. Pityriasis rosea C. Secondary syphilis D. Subacute cutaneous lupus E. Tinea versicolor 36 12 7/1/2013 Pityriasis Rosea • Common • Herald patch: 1 week earlier, larger plaque • Widespread, symmetrical oval pink papules and plaques with a central scale • Central trunk and back (Christmas tree pattern) • Mimics the rash of secondary syphilis – CHECK RPR 37 Next case 38 Subacute Cutaneous LE (SCLE) • Women aged 15-40 • 50% meet ARA criteria for SLE, only 10% severe • Renal or CNS disease rare = good prognosis • 80% ANA positive • Positive Ro/SSA – Neonatal heart block is risk • Photosensitive – Ro correlates with photosensitivity 39 13 7/1/2013 Subacute Cutaneous LE Skin Lesions • Papulosquamous: Resembles psoriasis • Annular • Sun-exposed areas • Face, V-neck chest, and back • Heals without scarring • (unlike discoid LE) 40 Case 5 • 55 yr old male • COPD, HTN, h/o psoriasis • Fever, shaking chills, and diffuse erythema (erythroderma) • Meds: – ACE inhibitor x 3 months – 1 week of pulsed prednisone with rapid taper for COPD flare 41 42 14 7/1/2013 Case 5, Question 1 The most likely diagnosis is: A. Drug eruption due to ACE inhibitor B. Paraneoplastic syndrome due to non-small cell lung cancer C. Sézary syndrome (cutaneous T-cell lymphoma) D. Flare of psoriasis due to prednisone taper E. Staphylococcal Scalded Skin Syndrome 43 Case 5, Question 1 The most likely diagnosis is: A. Drug eruption due to ACE inhibitor B. Paraneoplastic syndrome due to non-small cell lung cancer C. Sézary syndrome (cutaneous T-cell lymphoma) D. Flare of psoriasis due to prednisone taper E. Staphylococcal Scalded Skin Syndrome 44 Pustular Psoriasis • Commonly drug-induced • Corticosteroid taper • Psoriasis flare + pustules • Can be life threatening – High cardiac output state – Electrolyte imbalance – Respiratory distress – Temperature dysregulation 45 15 7/1/2013 Psoriasis Comorbidities • Recent evidence links severe psoriasis with – Arthritis – Cardiovascular disease (including MI) – Hypertension – Obesity – Diabetes – Metabolic syndrome – Malignancies • Lymphomas, SCCs, ? Solid organ malignancies – Higher mortality – Poor quality of life 46 Psoriasis 47 48 16 7/1/2013 Psoriasis Aggravators • Medications • Sunburn – Systemic steroids • Severe life stress – Beta blockers •HIV – Lithium – Up to 6% of patients – Hydroxychloroquine with AIDS develop psoriasis • Strep infections • Alcohol for some – Guttate psoriasis (children) • Smoking for some • Trauma 49 Treatment for Psoriasis • Topical therapy – Steroid ointment (start mid-potency) – Calcipotriene ointment –Tar • Phototherapy – PUVA: psoralens + UVA • Systemic therapy – Acitretin (oral retinoid) – Methotrexate, cyclosporine – Biologics • etanercept, infliximab, adalimumab (TNF alpha blockade) • ustekinumab (IL-12, IL-23 blockade) **Systemic steroids are NOT on this list!** 50 Case 6 • 42 yo HIV+ male admitted to ICU • Severely hypotensive IV fluids, norepinephrine • ?Sepsis antibiotics are started • History of taking TMP/SMX for UTI • 24 hrs after admission: • febrile • rash, rapidly progressive • skin is painful • gritty sensation in eyes • oral pain, difficulty swallowing 51 17 7/1/2013 52 Case 6, Question 1 The most likely diagnosis is: A. Drug Eruption B. Staphylococcal Scalded Skin Syndrome C. Autoimmune Blistering Disorder D. Toxic Shock Syndrome E. Severe viral exanthem 53 Case 6, Question 1 The most likely diagnosis is: A. Drug Eruption B. Staphylococcal Scalded Skin Syndrome C. Autoimmune Blistering Disorder D. Toxic Shock Syndrome E. Severe viral exanthem Toxic epidermal necrolysis (TEN) 54 18 7/1/2013 Case 6, Question
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